Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liver biopsies were studied in 47 cases representing various histiocytosis syndromes. These included 32 cases of hemophagocytic syndrome, 11 cases of Langerhans cell histiocysis (LCH), and 4 cases of other histiocytic disorders. All cases of hemophagocytic syndrome, except one with cytomegalovirus infection, displayed portal lymphohistiocytic infiltrates dominated by T lymphocytes. Activation of the hepatic mononuclear phagocytic system (MPS), evidenced by enlarged von Kupffer cells, some of which were hemophagocytic, was seen in 28 cases of hemophagocytic syndrome. Endothelial enlargement, minor degrees of hepatocellular degeneration, and steatosis were also noted. Ten of the 11 cases of LCH also showed activation of the MPS. It was the only lesion in two biopsies. Seven cases demonstrated nonspecific "triaditis." In three this was associated with cholangiocentric and random acinar histiocytic lesions. Evidence of activation of the MPS was also observed in both cases of Rosai-Dorfman disease and was accompanied by acinar histiocytic lesions in one and triaditis in the other. Likewise, both cases of juvenile xanthogranuloma showed activation of the MPS and focal granulomatous lesions. It is concluded that activation of the MPS is a common feature of liver disease in histiocytosis syndromes and that hepatic enlargement may be the result of this process instead of, or in addition to, the liver lesions known to be featured in these disorders. Hepatic lesions of the various histiocytosis syndromes resemble typical lesions in other sites and, in some instances, are accompanied by nonspecific changes. These nonspecific changes may occur in the absence of lesions that are diagnostic or typical of the particular histiocytosis syndrome. The location and character of hepatic lesions are important factors in the significance of liver involvement.
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PMID:Histopathology of the liver in histiocytosis syndromes. 902 43

ALK-positive histiocytosis is a recently described rare histiocytic proliferative disorder of early infancy. When infants present with anemia, thrombocytopenia, and hepatosplenomegaly, this entity should be included in the differential diagnosis along with hemophagocytic lymphohistiocytosis, metabolic/storage diseases, hematopoietic malignancies, and autoimmune thrombocytopenia. We report 2 new cases of ALK-positive histiocytosis, one with kidney involvement and the other with extensive bone marrow involvement in addition to an overt liver disease. Renal involvement by ALK-positive histiocytosis has not been reported. The infiltrating histiocytes in this case showed Rosai-Dorfman disease-like morphology including emperipolesis. The histiocytes in the second case with extensive bone marrow involvement had foamy cytoplasm initially suggesting storage disease. Our 2 cases highlight previously unrecognized diversity of ALK-positive histiocytosis in clinical manifestation, organ involvement, and cytomorphologic features and further elucidate the diagnostic challenges of this rare entity.
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PMID:Expanding the Phenotype of ALK-positive Histiocytosis: A Report of 2 Cases. 2922 19